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Defects in t6A tRNA modification due to GON7 and YRDC mutations lead to Galloway-Mowat syndrome

Authors :
Christelle Arrondel
Sophia Missoury
Rozemarijn Snoek
Julie Patat
Giulia Menara
Bruno Collinet
Dominique Liger
Dominique Durand
Olivier Gribouval
Olivia Boyer
Laurine Buscara
Gaëlle Martin
Eduardo Machuca
Fabien Nevo
Ewen Lescop
Daniela A. Braun
Anne-Claire Boschat
Sylvia Sanquer
Ida Chiara Guerrera
Patrick Revy
Mélanie Parisot
Cécile Masson
Nathalie Boddaert
Marina Charbit
Stéphane Decramer
Robert Novo
Marie-Alice Macher
Bruno Ranchin
Justine Bacchetta
Audrey Laurent
Sophie Collardeau-Frachon
Albertien M. van Eerde
Friedhelm Hildebrandt
Daniella Magen
Corinne Antignac
Herman van Tilbeurgh
Géraldine Mollet
Source :
Nature Communications, Vol 10, Iss 1, Pp 1-13 (2019)
Publication Year :
2019
Publisher :
Nature Portfolio, 2019.

Abstract

The biosynthesis of N6-threonylcarbamoylated adenosine 37 in tRNA (t6A) involves the YRDC enzyme and the KEOPS complex. Here, the authors report mutations in YRDC and the KEOPS component GON7 in Galloway-Mowat syndrome and determine the crystal structure of a GON7-containg subcomplex that suggests a role in KEOPS complex stability.

Subjects

Subjects :
Science

Details

Language :
English
ISSN :
20411723
Volume :
10
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Nature Communications
Publication Type :
Academic Journal
Accession number :
edsdoj.77cd12b7d5974fda8810f1a228293257
Document Type :
article
Full Text :
https://doi.org/10.1038/s41467-019-11951-x